Posttraumatic epilepsy and acute intermittent porphyria: effects of phenytoin, carbamazepine, and clonazepam.

A patient with uncontrolled posttraumatic epilepsy and acute intermittent prophyria was subjected to successive therapeutic trials with phenytoin, carbamazepine, and clonazepam, while eating an adequate diet. Both phenytoin and carbamazepine treatments caused significant increases in porphobilinogen excretion and appeared to induce acute porphyric attacks. In contrast, treatment with clonazepam under rigid dietary control for 10 days caused no increase in porphilbinogen excretion. During the subsequent 7 months of treatment with clonazepam, neither seizures nor porphyric attacks recurred. These findings suggest that clonazepam may be a safe and effective treatment for chronic or severe generalized seizure disorders in patients with acute intermittent porphyria.

Endoscopy or radiography?--The patient's choice. Prospective comparative survey of patient acceptability of upper gastrointestinal endoscopy and radiography.

Patient acceptability of endoscopy and the double-contrast barium meal test was compared in a prospective randomized fashion. Ninety-six inpatients were interviewed with a standardized questionnaire within 24 hours of completion of double-contrast barium meal test and endoscopy, after recovery from administered sedation. Twenty-five percent of patients reported moderate to severe discomfort with each procedure. The levels of discomfort reported were similar for both procedures. The majority of patients would readily undergo a repeated procedure, if requested. There was a tendency (p less than 0.1) for patients to prefer endoscopy overall. No specific patient group that preferred one procedure over the other could be identified, although older patients (more than 55 years) showed a tendency to prefer endoscopy. The choice of one procedure over the other should no longer be based on the presumed superior acceptability of the double-contrast barium meal test.

Urinary calculi associated with purine metabolism. Uric acid nephrolithiasis.

Uric acid stones are the consequences of abnormalities in purine metabolism, urate/uric acid renal handling, or excess dietary protein. Treatment is aimed at preventing additional formation by decreasing the degree of urate and uric acid supersaturation in urine, by altering diet, fluid intake, and the urine pH, and by blocking steps in uric acid production.

Initial assessment of the patient with hypertension.

The initial assessment of a patient with hypertension can easily be done in a primary-care setting. The goals of the examination are to determine whether the patient is truly hypertensive and, if so, the severity of the hypertension, the degree of target-organ involvement, the presence of curable causes of hypertension, the patient's overall cardiovascular risk profile, and the patient's understanding and willingness to adopt necessary life-style changes and comply with treatment.

A rare complication of aortic valve endocarditis: diagnosis with transesophageal echocardiography.

Rupture of the membranous interventricular septum resulting in a left ventricular to right atrial shunt is a rare complication of endocarditis. Early recognition of this complication is essential because these patients require early surgical intervention. We report a case of a left ventricular-right atrial shunt complicating aortic valve endocarditis in which transesophageal echocardiography was used to accurately diagnose the lesion before surgery. We will discuss the role of transesophageal echocardiography in the diagnosis of this lesion and will review the existing literature pertaining to this rare complication.

Cross-linking CD40 on human B cell precursors inhibits or enhances growth depending on the stage of development and the IL costimulus.

The function of the cell surface molecule CD40 on B cell precursors (BCP) is not well understood. We now report studies using the L cell/CD40 system (anti-CD40 mAb immobilized on CD32+ mouse L cells) to assess the potential function of CD40 during human B cell ontogeny. Stimulation of human B lineage cells with IL-4 in the L cell/CD40 system yielded a hierarchy of responsiveness: high density tonsillar B cells > fetal splenic B cells > fetal bone marrow surface Ig+ immature B cells > fetal bone marrow surface Ig- BCP. Using a microsphere/flow cytometry growth quantitation assay, we found that substituting IL-3 for IL-4 in the L cell/CD40 system provided a stronger growth stimulus for fetal bone marrow BCP and immature B cells. We also found that FACS-purified fetal bone marrow CD10+/CD34+/CD40+/cytoplasmic mu- pro-B cells responded maximally to IL-3 plus IL-7. Surprisingly, anti-CD40 inhibited the pro-B cell response to IL-7. In contrast, FACS-purified fetal bone marrow CD10+/CD34-/CD40+/cytoplasmic mu+ pre-B cells were essentially nonresponsive to IL-3, IL-7, or anti-CD40 alone, but were uniquely responsive to IL-3 plus anti-CD40. B-lineage cells derived after 14 days from IL-7-stimulated pro-B cells were predominantly CD19+/L chain-, whereas pre-B cells stimulated with IL-3 plus anti-CD40 were predominantly CD19+/L chain+. The L chain+ cells from pre-B cell cultures were both mu+/delta+ and mu-/delta+. Our results demonstrate that the response to CD40 signaling depends upon the BCP developmental stage and the IL costimulus, and indicate that normal human pro-B cells and pre-B cells have different growth factor requirements.

A prospective, randomized controlled trial of chronic esophageal variceal sclerotherapy.

The results of a prospective, randomized controlled trial of chronic esophageal variceal sclerotherapy conducted over a 38-month period are presented. One-hundred twenty patients were randomized following variceal bleeding, 63 to esophageal variceal sclerotherapy and 57 to control. Mean follow-up was similar in both groups (esophageal variceal sclerotherapy, 12.5 +/- 8.8 months; control, 14.9 +/- 6.6 months). Twenty-one percent of the patients in each group were lost to follow-up. Esophageal variceal sclerotherapy decreased rebleeding as evidenced by a decrease in the mean bleeding risk factor, transfusion requirement and by an increase in bleeding free interval; differences between the treated and control groups in these parameters were especially significant after variceal obliteration. A high incidence of asymptomatic ulceration and low frequency of strictures were notable effects of esophageal variceal sclerotherapy. Cumulative life table analysis revealed no differences in survival between esophageal variceal sclerotherapy and control groups. However, when patients who received portal-systemic shunt surgery (esophageal variceal sclerotherapy, 6%; control, 28%) were removed from the analysis at the time the shunt surgery was performed (defining the shunt as an endpoint, a significant difference in survival (p less than 0.05, F ratios) in favor of esophageal variceal sclerotherapy was observed.

Acute esophageal variceal sclerotherapy. Results of a prospective randomized controlled trial.

Within 48 hours of variceal hemorrhage, 82 patients were randomly assigned to conventional treatment including balloon tamponade or to conventional treatment supplemented by sclerotherapy. The prerandomization general clinical characteristics of the two groups were similar. Seventy-nine percent of patients were alcoholic and 57% were in Child's class C. In the sclerotherapy group of 44 patients, sclerotherapy was performed twice in 28 patients and thrice in 13 patients over the two weeks of follow-up. The number of patients who rebled was significantly lower in the sclerotherapy group than in the group treated conventionally (23% vs 53%). The number of bleeding episodes also was significantly lower in the sclerotherapy group (15 vs 32). Moreover, blood transfusion requirements were significantly decreased in the sclerotherapy group. This was true even for patients who were bleeding at the time of randomization or who were in Child's class C, but this primarily was because fewer patients who underwent sclerotherapy (10 of 24 vs 18 of 23) required transfusions. Although there was no difference in survival between the two groups, we conclude that sclerotherapy is better than conventional treatment in the acute management of variceal bleeding.

A giant renal artery aneurysm diagnosed post partum.

We report a case of a 5.8 cm. right renal artery aneurysm diagnosed intact 8 weeks post partum. Rupture of a renal arterial aneurysm during pregnancy is a rare but well described catastrophic event. There are no previous reports of an intact renal artery aneurysm diagnosed either ante partum or post partum. To our knowledge, this also represents the largest reported renal artery aneurysm. The aneurysm was repaired successfully with kidney salvage and closure of the fistulous connection to the renal vein.

Double-contrast barium meal and upper gastrointestinal endoscopy. A comparative study.

One hundred randomly selected inpatients were examined with both double-contrast barium meal and endoscopy in a blinded prospective fashion. All studies were done by staff personnel, with equal clinical information available to both the radiologist and endoscopist. The final diagnosis was made by a review committee of participating radiologists and endoscopists. Endoscopy was more sensitive (92% versus 54%, p less than 0.001) and specific (100% versus 91%, p less than 0.05) than the double-contrast barium meal. Both procedures significantly affected the clinical outcome of the patient, the effect of endoscopy being significantly greater than that of the double-contrast barium meal. Although errors with the barium study related predominantly to an inability to show subtle lesions, poor patient cooperation and perceptual and technical failures were additional significant factors. Endoscopy is recommended for certain groups of patients.